KAPOSI'S SARCOMA

By Gary M. White, MD

Kaposi's sarcoma A vascular papule on the foot, classic type (non-HIV).


Kaposi's sarcoma (KS) is a vascular proliferation of lymphatic endothelial cells that is related to infection by the human herpesvirus 8. Broadly speaking, there are two types of Kaposi's sarcoma (KS), the classic type, occurring in men of Southern or Eastern European descent and KS in immunocompromised patients, e.g., HIV, organ transplant.

Clinical

Classic KS usually presents as a reddish-blue to purple papulonodule or plaque beginning on the toe or sole. Progression is usually slow, but ultimately, almost any area of the body may be involved, especially the gastrointestinal tract.

HIV-related (and immunocompromised-related) KS usually presents as an asymptomatic, erythematous macule which later becomes raised and violaceous. Lesions are often ovoid following lines of cleavage. Lesions are common on the trunk, extremities, and oral cavity (where the palate is preferred). Koebnerization may occur.

Treatment

HAART

Highly active antiretroviral therapy (HAART) is indispensable in the treatment of the AIDS-related KS, alone or in combination with systemic and/or local therapy. HAART can prevent the occurrence of KS or can induce regression of KS lesions. Watch for a rise in the CD4 count to coincide with improvement of the lesions.

Topical Timolol

Topical timolol gel 0.1% BID completely cleared 3 patients in 4-5 weeks [JAAD 2017;76;153].

Sirolimus (Rapamycin)

Switching to sirolimus (e.g., from cyclosporine) can be very beneficial. In one report of 15 organ transplant patients with KS, switching to sirolimus cleared the KS [N Engl J Med. 2005 Mar 31;352(13):1317-23].

Local Destruction

Local treatment is usually done for limited disease. Elimination of any clinically apparent lesion is the goal. Clear margins are not required as the process is presumed systemic. Lesions may be frozen, curetted, or injected. One recipe for intralesional vinblastine is vinblastine 0.2 mg/cc injected intralesionally 0.1-0.3 cc/lesion monthly as needed. Each lesion is injected until it blanches.

Topical Imiquimod

Imiquimod. e.g. 5% cream topically has been used in KS patients that are HIV positive, HIV negative and status post renal transplant. It has been used 3/week, nightly and nightly occluded. Its response is variable, ranging from complete clearing to partial to no effect. Some cases clear clinically but show evidence histologically. See for example, nightly occluded imiquimod 5% cream on the sole of a non HIV patient with KS nearly cleared after 3 months [J Dermatol Case Rep. 2012 June 30].

Topical Sirolimus

Topical sirolimus in an immunocompetent patient achieved clinical and histologic healing after 16 weeks of treatment [Dermatol Ther. 2014 Oct 14].

Panretin

Alitretinoin gel 0.1% (Panretin) was mildly effective in local control of the lesions of Kaposi's sarcoma in a prospective multicenter randomized trial in which the substance was used two times a day for 12 weeks. Overall response rate was 37%.

Radiation Therapy

Radiation therapy can be effective but is usually reserved for localized or intraoral lesions.

Chemotherapy

Highly active antiretroviral therapy (HAART) plus chemotherapy may be beneficial in reducing disease progression compared to HAART alone in patients with severe or progressive Kaposi's sarcoma [Cochrane Database Syst Rev. 2014 Aug 13;8]. Several drugs, such as vincristine, vinblastine, etoposide, bleomycin, docetaxel, and paclitaxel, can be administered. Liposomal anthracyclines, doxorubicin, and daunorubicin can be very effective in the treatment of advanced KS.

Intraoral Lesions

Intraoral lesions have responded to intralesional therapy (e.g. vinblastine or 3% sodium tetradactyl sulfate), and radiation therapy.

Miscellaneous

There is some evidence that patients with KS should not be treated with phototherapy (e.g., for pruritus) as their KS can worsen.

Additional Pictures

The oral cavity is a common location.
Kaposi's sarcoma Kaposi's sarcoma

Kaposi's sarcoma Kaposi's sarcoma Kaposi's sarcoma Kaposi's sarcoma Kaposi's sarcoma Kaposi's sarcoma

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